Introduction: Evans syndrome (ES) is a rare autoimmune hemato- logical entity characterized by simultaneous or sequential occurrence of autoimmune ...
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Abstracts
ID: 820 Evans syndrome: About 11 cases J. Anouna, B. Achourb, A. Rezguia, H. Regaiegb, N. Amrib, E. Bouslemab, N. Ben Sayedb, N. Saidb, Y. Ben Youssefb, A. Khelifb a
Department of Internal Medicine, Sahloul Hospital, Sousse, Tunisia Department of Hematology, Farhat Hached Hospital, Sousse, Tunisia
b
Introduction: Evans syndrome (ES) is a rare autoimmune hematological entity characterized by simultaneous or sequential occurrence of autoimmune hemolytic anemia and thrombocytopenia, sometimes associated with autoimmune neutropenia. Although in most cases the Evans syndrome is idiopathic, it may be secondary to a lymphoproliferative disorder or system disease. Purpose: Study the epidemiological, clinical, paraclinical and therapeutic specifications of Evans syndrome. Materials and methods: This is a retrospective analytic study about 11 cases of Evans syndrome collected in the department of hematology at Farhad Hached University Hospital and the internal medicine department of Sahloul Hospital of Sousse over a period of 09 years. Results: There were 6 men and 5 women (sex ratio = 1.2), the median age was 50.72 years (28 years–77 years). All patients presented an anemic syndrome (pallor = 100%, asthenia = 89%) and 3 had bleeding symptoms. At physical examination, 3 patients had icterus, 5 had splenomegaly, 2 had lymphadenopathies and two had disseminated intravascular coagulation (DIVC). Biologically, there was regenerative normocytic anemia in 81.8% with neutropenia in 45% of patients and severe thrombocytopenia (b20000) in 2 patients. High levels of bilirubin were found in 45.45% of cases and for LDH in 63.63%. Direct Coombs test has confirmed the autoimmune hemolytic anemia. It was positive for IgG (6 cases), C3 (2 cases), IgG + C3 (2 cases) and cryoagglutinins (1 case). Evans syndrome was secondary in 6 patients to: a megaloblastic anemia, a chronic lymphocytic leukemia, a large Bcell lymphoma, a Systemic lupus erythematosus, a Sharp syndrome and an Ankylosing spondylarthritis. Blood transfusion was indicated in 33% of patients. All patients, except the case of cryoagglutinins AIHA, underwent treatment with corticosteroids for an average of 6 months. Immunosuppressant treatment was prescribed for one patient and splenectomy was performed in one patient. We were thus able to notice a complete remission in 72.72% of cases, chronicity in 18.18% of cases and death in 9% of cases. Conclusion: Evans syndrome is the result of immune dysregulation resulting in a breach of one or more hematopoietic lineages. It can be life-threatening and requires adequate and rapid management. doi:10.1016/j.ejim.2013.08.454
ID: 828 Megaloblastic anemia as a differential diagnosis in pancytopenia C.M. Pereira, S. Moura, E. Pinelo, P. Vaz Department of Internal Medicine, Unidade Local de Saúde do Nordeste, Bragança, Portugal
Introduction: Pancytopenia is considered a reduction of all lineages of blood cells. Megaloblastic anemia is one of its several causes, which is a group of disorders characterized by the presence of different morphological aspects of the maturing cells in the bone marrow associated with ineffective erythropoiesis. Objectives: Description of a clinical case. Case: We present a case of a 39 years old male, ex-drug addict (cocaine and heroin), with severe drinking habits, that was brought to the emergency room for extreme fatigue and palpitations at rest with three months of evolution. He complained about anorexia and
frequent epistaxis in the last week too. At physical examination, the patient was awake and oriented; his vital signs were temperature 37 °C, blood pressure 85/44 mm Hg, heart rate 115 bpm, and respiratory rate 24/min, without signs of respiratory distress. The patient was extremely pale and dehydrated, with no rash, absence of petechiae, purpura or hemorrhagic manifestations; no palpable lymphadenopathy; cardiopulmonary auscultation had no significant alterations; painless abdomen with hepatosplenomegaly without palpable masses; and severe generalized edema. From his laboratory tests we highlight a severe pancytopenia: macrocytic anemia (Hb 2.8 g/dL), thrombocytopenia (16,000 platelets/μL) and leukopenia (2650 leukocytes/μL) with decreases in all lineages of leukocytes. Allied to these changes, we found liver cytolysis and a very high LDH (5341). Abdominal CT showed liver cirrhosis with splenomegaly related with portal hypertension. In the study of anemia, a severe deficiency of folic acid was discovered. A peripheral blood smear and bone marrow were done, consistent with megaloblastic anemia. Therefore, we were before a chronic alcoholic liver disease associated with a severe nutritional deficiency. The patient initiated oral supplementation of folate and B12 vitamin, with good response: normalization of all cell lines with three months of treatment. Conclusions: This case reinforces the importance of the exclusion of nutritional deficits in the differential diagnosis of anemia and pancytopenia, even in developed countries. doi:10.1016/j.ejim.2013.08.455
ID: 854 Superior vena cava syndrome in primary mediastinal B-cell lymphoma L. Ruzickovaa, C. Canhab, L. Geraldesb, P. Césara, J. Cardab, N. Costaa, B. Barbosab, M. Veríssimob, J. Costab a
Haematology, Coimbra's University Hospital, Coimbra, Portugal Internal Medicine, Coimbra University Hospital, Coimbra, Portugal
b
Introduction: Superior vena cava syndrome (SVCS) is a group of symptoms that occurs when vena cava superior is partly or completely blocked. Obstruction can occur due to external pressure, involvement of the vessel by tumour tissue, or thrombotic event. Most SVC syndromes are associated with malignant disease. Primary Mediastinal B-cell lymphoma is a distinctive subtype of Non-Hodgkin lymphoma with specific clinicopathologic aspects and aggressive behaviour. This malignancy is characterized by rapid growth, large bulk, and early local and extranodal extension in the absence of generalized adenopathy. Most patients demonstrate symptoms, often present for only several days, from a rapidly enlarging mediastinal mass. It is predominantly a disease of young adults, particularly women. Superior vena cava syndrome is common, as is involvement of the pericardium. Case summary: We present the case of 33-year-old, Caucasian woman with medical history of recently diagnosed hypothyroidism medicated with Levothyroxin, who presented to the Emergency Department with dyspnea, swelling and flushing of the face and neck and upper extremities which increased overnight when she lay in a horizontal position. In addition, she had developed odynophagy and low back pain and bilateral axillar pain within the course of approximately one week. Ultrasound exam shows multiple bilateral ganglionar formations along the cervical glands chains, without pathology of thyroide gland or other structures. Computed tomography was performed disclosing anterior mediastinal mass (10 × 9.5 × 4.5 cm) involving superior cava vein, causing severe obstruction on the level of the right atrium entry, small adenopathies on the periphery of the mass and small pleural effusion. Histopathological examination of the mass revealed sclerosing mediastinal B- cell lymphoma (CD3 and CD20 histochemical positivity). PET-
Abstracts
Tomography revealed supradiapharagmatic ganglionar involvement without other hypermethabolic alterations. Initial medical management consisted of pain relief medication and corticotherapy with quick symptomatic relieve. At present, the patient is undergoing chemotherapy. Discussion: SVCS is considered an emergency, however, its presentation is rarely an acute life-threatening event. As the effectiveness of therapy depends on underlying disease, considering the definitive diagnosis may be more important than the actual care when making therapeutic decisions. Conclusion: Facial swelling and respiratory complaints are frequent symptoms in Emergency Department patients. They requires quick and complex diagnostic evaluation including differential diagnosis of SVCS. The malignant cause of SVCS should be always considered even in younger patients.
doi:10.1016/j.ejim.2013.08.456
ID: 860 Multiple myeloma in the elderly: Epidemiology, diagnosis and treatment of 35 cases J. Anouna, H. Regaiegb, B. Achourb, E. Bouslemab, N. Ben Sayedb, N. Amrib, N. Saidb, Y. Ben Youssefb, A. Khelifb a
Department of Internal Medicine, Sahloul Hospital, Sousse, Tunisia Department of Hematology, Farhat Hached Hospital, Sousse, Tunisia
b
Introduction: Multiple myeloma is a lymphoproliferative disorder characterized by proliferation of plasma cells responsible for monoclonal infiltration of bone marrow and the synthesis of a complete or incomplete immunoglobulin. It is usually seen in patients over 60 years of age. The association with other diseases is common. The melphalan-prednisone (MP) has been for decades the standard treatment of multiple myeloma (MM) in the elderly. Our work aims to demonstrate the contribution of thalidomide in the treatment of MM in the elderly. Methods: Our work is a retrospective study including 35 patients aged over 65 years with MM monitored and treated according to the Tunisian national protocol in the Hematology department of Farhat Hached Hospital, Sousse, during the period extending from March 2006 to August 2012. The evaluation of the response at the end of induction therapy was made according to the criteria of the international myeloma working group (IMWGS). The analysis of survival without events (SSE) and overall survival (OS) was performed according to the Kaplan Meir. Results: Thirty-five patients over 65 years (66–85 years) were followed in our hematology department with a sex ratio of 1.6 (21 men and 14 women). The general condition was good in 20 patients. The presence of comorbidity was present in 18 patients.31 patients were stage III according to the classification of salmon and Durie with renal failure in 10 cases. These patients received initial chemotherapy type MP or MPT. 18 patients received induction therapy with MP. Partial response (PR) was observed in 12 patients and no complete response (CR) was obtained. OS was 38% at 28 months. 13 patients received induction therapy with MPT. At least a PR was obtained in 6 cases with a CR in 3 cases. OS was 26% at 50 months. Discussion: Thalidomide was the first agent used in combination with the MP. Five randomized trials comparing this combination with melphalan-prednisone in the treatment of MM in patients older than 65 years have demonstrated the superiority of MPT protocol in terms of response, SSE and OS. Conclusion: Until now, multiple myeloma has remained an incurable disease. Its occurrence in the elderly represents a therapeutic problem, mainly due to the patient's age and comorbidity but also to prognostic factors: high tumor burden, general condition.
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doi:10.1016/j.ejim.2013.08.457
ID: 889 Hypereosinophilia — The only sign of cancer J. Lino, M. Alves, R. Gomes, A. Silva, Z. Soares, T. Camacho, B. Ferreira, C. Louro, V. Goric, J. Barata Internal Medicine Department, Vila Franca de Xira Hospital, Vila Franca de Xira, Portugal
Objectives: The authors present a case of cancer without symptomatology and the single manifestation was a hypereosinophilic change. We want to show how the slow progression of disease and the invasive exams without diagnosis can be very demotivational for the patient. Material and methods: A 40-yearold man, he's healthy but has with a history of alcoholism. He was referenced at our center in 2007 by having hypereosinophilia (64%). Results: The myelogram and the bone biopsy revealed hypereosinophilic syndrome, suggesting a molecular and cytogenetic study. The patient, however, did not attend the follow-up. After three years he return, sent by the family doctor, for maintenance of hypereosinophilia. Abdominal computed tomography revealed hepatosplenomegaly with ascites and right pleural effusion, it may be a result from lymphoproliferative disease. No other significant changes were seen. We repeated the bone biopsy that identified chronic myeloproliferative neoplasy with eosinophilia with morphological features that suggested a chronic myelomonocytic leukemia with marked eosinophilia. Discussion and conclusion: As this is a pathology, in its early stages it has no symptoms, and its only manifestation is an analytic change. The patient, after having invasive diagnostic techniques and without a definitive result, was demotivated to investigate his disease. It is also important to note that, although the first tests did not reveal significant changes, it is sometimes necessary to repeat them and always clarify the patient in order to make in cooperate with his doctor.
doi:10.1016/j.ejim.2013.08.458
ID: 923 Hemorrhagic bullae — An alarm signal L. Santosa, J. Queiróa, J. Carlosb, A. Figueiredoa a
Internal Medicine, Hospital Geral — Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal b Haematology, Hospital Geral — Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
The non-traumatic hemorrhagic bullous lesions are rare and a sign of serious illness in most clinical situations, as it is associating with severe disorders from coagulation or platelets, whose underlying cause is not always easy to ascertain, but often related with autoimmune disorders or infectious. We report the clinical case of a patient aged 81, who appealed to the Emergency Room by presenting hemorrhagic bullous lesions on the oral mucosa, of sudden onset, associated with purpura on the legs, with the same trend, rapidly progressive in about 1 day. There was no history of epistaxis, hematemesis, melena, or hematuria. Previous clinical records of relevance only showed hypertension. She denied infectious process or history of trauma. On physical examination, no lymphadenopathy or organomegaly. Analytically with absolute thrombocytopenia (0.0), without anemia (13.4 g/dl)
